Monday, October 8, 2007

Huntington's Disease: its Symptoms and Effects

QUESTION: Can you tell me about Huntington's Disease? Where does it come
from, and what are its symptoms and effects?
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ANSWER: It is sometimes called Huntington's Chorea or more precisely,
progressive hereditary chorea. It is an inherited disease, carried by a
single dominant gene, which means that if present in the genetic makeup of an
individual, it will always cause the disease. The tragedy of Huntington's is
that the disease doesn't begin to manifest itself until middle age (35-50
years old) when many sufferers have already married and had children. The
odds are that 1 out of 2 children will also have the disease which strikes men
and women equally. It begins in an insidious fashion, striking at the mind
and body. The patient's personality changes, as he/she becomes irrational or
apathetic, or a manic-depressive or schizophrenia-like illness may develop
before the beginning of the muscle problems. The patient cannot walk, may
have difficulty in swallowing, eventually cannot care for themselves as the
choreic (spastic, involuntary, irregular) movements of the muscles become
worse. The disease continues to the inevitable end, as there are no cures or
treatments, and medications can only partially reduce the symptoms. The
recent discovery of a genetic marker for Huntington's permits physicians to
offer considered counsel. Members of families in which the gene is present
are generally advised to avoid child bearing, but should seek genetic
counseling to aid them in the decision which they alone can make.

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